Lymphangiomyomatosis

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Template:Lymphangiomyomatosis Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Varun Kumar, M.B.B.S. [2]

Synonyms and keywords: Lymphangioleiomyomatosis; LAM

Overview

Lymphangiomyocytosis is defined as a multifocal neoplasm with differentiation of the perivascular epithelioid cell and has a female prepondrance, especially females of child-bearing age.

Characteristic Features

Lymphangiomyocytosis is characterized by the presence of smooth muscle and epithelioid cells and by the proliferation of lymphatic vessels.

Sites of Involvement

  • Lungs,
  • Mediastinum, and
  • Retroperitoneum.

Presentation

It usually presents with chylous pleural effusion or ascites.

Diagnosis

Chest X-ray

Reference

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